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Correlation between quantitative whole-body muscle magnetic resonance imaging and clinical muscle weakness in Pompe disease.

Publication ,  Journal Article
Horvath, JJ; Austin, SL; Case, LE; Greene, KB; Jones, HN; Soher, BJ; Kishnani, PS; Bashir, MR
Published in: Muscle Nerve
May 2015

INTRODUCTION: Previous examination of whole-body muscle involvement in Pompe disease has been limited to physical examination and/or qualitative magnetic resonance imaging (MRI). In this study we assess the feasibility of quantitative proton-density fat-fraction (PDFF) whole-body MRI in late-onset Pompe disease (LOPD) and compare the results with manual muscle testing. METHODS: Seven LOPD patients and 11 disease-free controls underwent whole-body PDFF MRI. Quantitative MR muscle group assessments were compared with physical testing of muscle groups. RESULTS: The 95% upper limits of confidence intervals for muscle groups were 4.9-12.6% in controls and 6.8-76.4% in LOPD patients. LOPD patients showed severe and consistent tongue and axial muscle group involvement, with less marked involvement of peripheral musculature. MRI was more sensitive than physical examination for detection of abnormality in multiple muscle groups. CONCLUSION: This integrated, quantitative approach to muscle assessment provides more detailed data than physical examination and may have clinical utility for monitoring disease progression and treatment response.

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Published In

Muscle Nerve

DOI

EISSN

1097-4598

Publication Date

May 2015

Volume

51

Issue

5

Start / End Page

722 / 730

Location

United States

Related Subject Headings

  • Whole Body Imaging
  • Severity of Illness Index
  • Sensitivity and Specificity
  • Pilot Projects
  • Physical Examination
  • Neurology & Neurosurgery
  • Muscle, Skeletal
  • Muscle Weakness
  • Muscle Strength
  • Middle Aged
 

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Horvath, J. J., Austin, S. L., Case, L. E., Greene, K. B., Jones, H. N., Soher, B. J., … Bashir, M. R. (2015). Correlation between quantitative whole-body muscle magnetic resonance imaging and clinical muscle weakness in Pompe disease. Muscle Nerve, 51(5), 722–730. https://doi.org/10.1002/mus.24437
Horvath, Jeffrey J., Stephanie L. Austin, Laura E. Case, Karla B. Greene, Harrison N. Jones, Brian J. Soher, Priya S. Kishnani, and Mustafa R. Bashir. “Correlation between quantitative whole-body muscle magnetic resonance imaging and clinical muscle weakness in Pompe disease.Muscle Nerve 51, no. 5 (May 2015): 722–30. https://doi.org/10.1002/mus.24437.
Horvath JJ, Austin SL, Case LE, Greene KB, Jones HN, Soher BJ, et al. Correlation between quantitative whole-body muscle magnetic resonance imaging and clinical muscle weakness in Pompe disease. Muscle Nerve. 2015 May;51(5):722–30.
Horvath, Jeffrey J., et al. “Correlation between quantitative whole-body muscle magnetic resonance imaging and clinical muscle weakness in Pompe disease.Muscle Nerve, vol. 51, no. 5, May 2015, pp. 722–30. Pubmed, doi:10.1002/mus.24437.
Horvath JJ, Austin SL, Case LE, Greene KB, Jones HN, Soher BJ, Kishnani PS, Bashir MR. Correlation between quantitative whole-body muscle magnetic resonance imaging and clinical muscle weakness in Pompe disease. Muscle Nerve. 2015 May;51(5):722–730.
Journal cover image

Published In

Muscle Nerve

DOI

EISSN

1097-4598

Publication Date

May 2015

Volume

51

Issue

5

Start / End Page

722 / 730

Location

United States

Related Subject Headings

  • Whole Body Imaging
  • Severity of Illness Index
  • Sensitivity and Specificity
  • Pilot Projects
  • Physical Examination
  • Neurology & Neurosurgery
  • Muscle, Skeletal
  • Muscle Weakness
  • Muscle Strength
  • Middle Aged